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Moyamoya disease symptoms and treatment


Moyamoya disease is a chronic progressive cerebrovascular disease in which blockage or stenosis occurs at the ends of both internal carotid arteries and at the beginning of the anterior and middle cerebral arteries corresponding to their branches. Moyamoya disease was named after Professor Suzuki in Japan in 1969. The abnormal shape of the blood vessels resembles that of rising smoke, so it is said that the Japanese name for it is ‘moyamoya’. Moyamoya disease is said to be about 10 times more common in Asians than in Westerners, and the incidence is particularly high in Korea and Japan. And it is known that the incidence rate is higher in women than in men.

The exact cause of moyamoya disease is not known. The theory that an autoimmune reaction occurs through infection and causes vasculitis, as well as genetic factors such as family history, and environmental factors are presumed to influence the occurrence, but nothing has been clearly confirmed yet. The onset age of moyamoya disease is high in both age groups under 10 years of age and between 30 and 40 years of age. Among them, the incidence rate is highest in children aged 7 to 9 years, and then it is reported to occur more frequently in adults in their 30s. Now, let’s take a closer look at the main symptoms of moyamoya disease, as well as various information on treatment and prevention.

Main symptoms of moyamoya disease

Symptoms of moyamoya disease vary depending on the onset of the disease. When it occurs in children, the amount of blood supplied to the brain decreases due to narrowing of the cerebral blood vessels, resulting in transient ischemic symptoms that temporarily impair brain function. When you cry, when your emotions become intense, when you blow a musical instrument or balloon, when breathing becomes rapid, cerebral blood vessel occlusion worsens, and paralysis symptoms appear in which one limb loses strength momentarily. This paralysis lasts for a few minutes and usually goes away on its own. In addition, symptoms such as speech disturbance, paresthesia, vomiting, nausea, and headache may also appear.

On the other hand, in adults, more often than in children, it appears as a symptom of cerebral hemorrhage caused by the bursting of abnormal blood vessels around the cerebral blood vessels. And depending on the area where the blood supply is insufficient, symptoms such as headaches and epileptic seizures, impaired consciousness, blurred vision, impaired speech, partial neuropathy, and decreased intelligence and memory may also occur.

Moyamoya disease does not show symptoms well in the early stages and has a characteristic that it progresses slowly. If the treatment period is delayed because symptoms appear as transient as this, it may leave permanent disability due to the occurrence of cerebral infarction and consciousness disturbance due to worsening of symptoms. In addition to the risk of developing symptoms such as generalized seizures and coma, in the worst case, it can have life-threatening and fatal consequences. In particular, hemorrhagic moyamoya disease in adults is reported to be a serious disease with a high degree of mortality that exceeds 25%.

Therefore, if the child has symptoms such as numbness or weakness in limbs after eating hot food, crying, or blowing an instrument or balloon, if symptoms such as epilepsy, stiff limbs and tremors appear It is important to get a diagnosis. It is known that there is no special method to prevent moyamoya disease, but if it is treated in a timely manner, a treatment result close to a full cure can be achieved, and as it is known that a normal life can be led without any major problems, it is possible to receive a detailed diagnosis and treatment as soon as possible. It can be said that it is very important to receive

Moyamoya disease treatment

1. Accurate diagnostic procedure through multiple tests

In moyamoya disease, various tests are performed to accurately check the progression of the disease, the need for surgery, and the timing of surgery. Diagnosis is made through various tests such as magnetic resonance angiography (MRA) and magnetic resonance imaging (MRI), as well as cerebral angiography to check the occlusion and stenosis formed at the distal end of the internal carotid artery and at the base of the cerebral blood vessels. In addition, tests such as positron emission tomography (PET) and radioisotope injection tomography (SPECT), which measure brain cell metabolic ability, may be performed. As it is known that such moyamoya disease is caused by a family history at a rate of about 15%, it is important to conduct periodic detailed examinations if there is moyamoya disease in the family.

2. Treatment according to the diagnosis result

Depending on the diagnosis result through the various tests mentioned above, a treatment process such as conservative drug treatment and surgical treatment is carried out. Immediately after the onset of acute cerebral ischemia symptoms, medical drug treatment is performed to relieve the symptoms, and treatment to protect the brain is carried out. However, since drugs to prevent or prevent disease progression have not yet been developed, in most cases, surgical treatment is considered first. These surgical treatments include direct vascular anastomosis, which increases blood flow by connecting scalp blood vessels and cerebral blood vessels, indirect vascular anastomosis, which places tissue containing scalp blood vessels on the brain surface to allow new blood vessels to grow inward, and direct and indirect vascular anastomosis. There is a combined vascular anastomosis that is performed together with vascular anastomosis.

Intermittent headaches and mild ischemia may occur after these multiple surgical treatments. Therefore, it is recommended to refrain from eating hot and spicy foods, blowing balloons or musical instruments, and various activities that cause symptoms, such as strenuous exercise.

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